A rare case of ascites
Aims
A 69-year-old gentleman presented with a 5-week history of abdominal distension. He had a past history of diabetes and myocardial infarction, and was an ex-smoker with no significant history of alcohol intake. Examination demonstrated a distended, non-tender abdomen with shifting dullness, no organomegaly and no signs of chronic liver disease.
Methods
Investigations included an ultrasound scan of the abdomen, an ascitic tap, a computed tomography (CT) of the abdomen/pelvis and a CT-guided biopsy.
Results
The ascitic tap revealed chylous ascites with a high serum ascites albumin gradient of >1.1 g/dL, indicating a non-peritoneal cause of ascites. Cytology revealed no evidence of malignancy. The CT of the abdomen revealed a mildly enhancing soft tissue mass, encasing the mesenteric and renal vessels and the upper abdominal aorta and also the left peritoneal space; appearances were suggestive of lymphoproliferative disorder. The CT-guided biopsy showed reactive changes consistent with retroperitoneal fibrosis.
Immunohistochemistry done at University College London showed no evidence of immunoglobulin G4 (IgG4) disease. The patient was commenced on prednisolone and azathioprine. He failed to tolerate azathioprine, which was then stopped. Treatment with prednisolone failed to slow the rate of reaccumulation of the ascites, and he continued to require frequent abdominal paracentesis.
Conclusions
Chylous ascites has rarely been reported as a presenting feature of retroperitoneal fibrosis. Retroperitoneal fibrosis may be idiopathic in 70% of cases or a secondary condition. The incidence of the idiopathic form is 0.1 per 100,000 person-years with a prevalence of 1.4 per 100,000 population. The primary method used for diagnosis of retroperitoneal fibrosis is CT; biopsies are performed in cases of unusual presentation and to exclude malignancy and IgG4-related pathology. Treatment of retroperitoneal fibrosis in most cases depends on whether it is idiopathic or secondary. The mainstay of treatment is corticosteroids; if there is no response, immunosuppressive therapy can be used. Case-series data exist, which show that high-dose corticosteroids like prednisolone are effective in reducing the chronic inflammatory response caused by retroperitoneal fibrosis; however, there is a high rate of recurrence once the steroids are withdrawn. Mycophenolate mofetil in addition to corticosteroids has been shown to reduce duration of steroid use without affecting the efficacy and reduces disease recurrence.
Conflict of interest statement
None declared.
- © Royal College of Physicians 2019. All rights reserved.
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